True ectopic ovary in the right iliac fossa mimicking acute appendicitis and associated with ipsilateral renal agenesis.

Ectopic ovarian tissue is a rare gynecologic condition. The presence of ectopic ovary may be accompanied by maldevelopments of the genital and urinary tract. We report an extremely rare case of a 39-year-old woman presenting with abdominal pain localized in the right lower quadrant. During the preoperative investigation and the exploratory laparotomy, an ectopic ovary in contact with the appendix accompanied by a single left kidney was found. The present report also includes a review of the related published work. To the best of our knowledge, this is one of the very few cases reported describing the co-occurrence of true ovarian ectopia and ipsilateral renal agenesis.